What are the other Names for this Condition? (Also known as/Synonyms)

• Crosti-Gianotti Syndrome
• Infantile Lichenoid Acrodermatitis
• Papular Acrodermatitis of Childhood

What is Gianotti-Crosti Syndrome? (Definition/Background Information)

• Gianotti-Crosti Syndrome (GCS) is a rare and benign skin rash on the arms and legs that is associated with several viral infections, commonly hepatitis B virus and Epstein-Barr virus infections. GCS is also reported after certain vaccinations
• The cause of formation of Gianotti-Crosti Syndrome is not well-understood. The skin condition is non-infectious (does not spread from one to another) and is mostly noted in childhood
• The signs and symptoms of Gianotti-Crosti Syndrome includes low-grade fever, sore throat, and swollen lymph nodes, apart from the skin symptoms. In most cases, no major complications are observed
• Generally, no treatment is required for Gianotti-Crosti Syndrome since the condition is known to resolve within 4 to 12 weeks. However, if required, topical medications may be administered for symptoms such as itchiness. The prognosis of GCS is typically good

Who gets Gianotti-Crosti Syndrome? (Age and Sex Distribution)

• Gianotti-Crosti Syndrome is a rare disorder that is mostly observed in children below age 10
• In children, both girls and boys are affected equally. In adults, a slight female preference is observed
• Worldwide, individuals of all racial and ethnic groups may be affected
• GCS is more common during spring and summer months

What are the Risk Factors for Gianotti-Crosti Syndrome? (Predisposing Factors)

The following are some known risk factors for Gianotti-Crosti Syndrome:

• An increased incidence is noted in children with atopic dermatitis
• Viral infections caused by the following pathogens (but not limited to): Epstein-Barr virus and hepatitis B infections are the most frequent causes
  • Adenovirus
  • Coxsackievirus
  • Cytomegalovirus (CMV)
  • Echovirus
  • Enteroviruses
  • Epstein-Barr virus (EBV)
  • Hepatitis A and B viruses (HAV and HBV)
  • Herpes simplex viruses (HSV)
  • Human herpes virus 6 (HHV-6)
  • Human immunodeficiency virus (HIV)
  • Influenza virus
  • Mumps virus
  • Parainfluenza virus
  • Parvovirus B19
  • Poxviruses
  • Respiratory syncytial virus (RSV)
  • Rotavirus
• Immune-compromised status (such as with HIV infection)
• It is also reported after vaccinations (with live serum) for the following conditions:
  • Bacillus Calmette-Guerin (BCG)
  • Diphtheria-pertussis-tetanus (DPT)
  • Hepatitis B
  • Influenza
  • Japanese encephalitis
  • Measles vaccine
  • Poliomyelitis

Currently, since no genetic factors are documented, a positive family history is not considered to be a risk factor for Gianotti-Crosti Syndrome.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Gianotti-Crosti Syndrome? (Etiology)

The cause of development of Gianotti-Crosti Syndrome is presently unknown.

• It may be immune-mediated (such as due to hypersensitive reaction to a prior infection)
• In some cases, a preceding infection may not be evident

GCS is not transmitted from one individual to another through physical contact or sharing of items.

What are the Signs and Symptoms of Gianotti-Crosti Syndrome?

The signs and symptoms of Gianotti-Crosti Syndrome may vary among affected individuals. It may be mild or severe and may include:

• Appearance of symmetrical rashes on the body
• The rashes appear as papules that are 1 to 10 mm in size
• The rashes are itchy, and the blisters bleed on scratching
• Hyperpigmentation and hypopigmentation may be noted in the area of rashes
• Skin rashes are more common in the arms, legs, and buttocks (arms more than the legs)
• The palms and soles may be affected
• Usually, the trunk and scalp are spared
• The rash appears abruptly/suddenly

Other symptoms related to the underlying condition may include:

• Fever, usually low-grade
• Sore throat (pharyngitis)
• Nasal congestion
• Wheezing
• Headache
• Cough
• Lymph node enlargement
• Enlarged liver and/or spleen (hepatosplenomegaly)

The signs and symptoms of Gianotti-Crosti Syndrome last for 2-4 weeks, rarely up to 12 weeks.

How is Gianotti-Crosti Syndrome Diagnosed?

The following tests and procedures may be performed to diagnose Gianotti-Crosti Syndrome:

• Complete physical examination and a thorough medical history evaluation
• Lab tests, if necessary
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: It is not usually required, but when performed, it is mostly done to exclude other causes of skin rashes

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Gianotti-Crosti Syndrome?

Complications in Gianotti-Crosti Syndrome are rare, but may include the following:

• Hemorrhagic lesions
• Development of secondary infections
• Scarring leading to cosmetic concerns

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Gianotti-Crosti Syndrome Treated?

In a majority of cases, Gianotti-Crosti Syndrome does not require any treatment since it is a benign and self-limited illness.

• Patient reassurance is important
• If required, medications may be prescribed for itchiness such as antihistamines and topical steroids
• If GCS is caused by an underlying infection (such as HBV or EBV), then suitable management of the underlying infection may be necessary

How can Gianotti-Crosti Syndrome be Prevented?

Gianotti-Crosti Syndrome may not be preventable since it is believed to occur as a reaction to an underlying condition.

• Restriction from attending school and community activities is not required. However, if an underlying viral infection exists, then isolation may be recommended by the healthcare provider
• Vaccination of children must be undertaken per pediatrician’s advice

What is the Prognosis of Gianotti-Crosti Syndrome? (Outcomes/Resolutions)

• The prognosis of Gianotti-Crosti Syndrome is generally good, since the condition resolves on its own within a few weeks
• It is reported that after the institution of the hepatitis B virus vaccine, the incidence of GCS has decreased

Additional and Relevant Useful Information for Gianotti-Crosti Syndrome:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/